Subcutaneous Panniculitic T-Cell Lymphoma in an Adolescent: A Case Report

Abstract

Rationale and Objectives: Subcutaneous panniculitic T-cell lymphoma (SPTCL) is a rare neoplasm accounting for less than 1% of pediatric Non-Hodgkin Lymphomas. It is most common in young adults with predominance of female cases at 0.5. Symptoms include multiple nodules involving the subcutaneous tissues of the extremities and trunk, neck and face. B symptoms such as fever, chills, night sweats and weight loss, have been reported. Rare extra-cutaneous manifestations include edema, involvement of the bone marrow, lymph nodes, liver, spleen, lungs and viscera. Laboratory abnormalities such as cytopenias and elevated lactate dehydrogenase have been recorded. Hemophagocytic syndrome (HPS) occurs in 33% of cases, which were correlated with fatal outcome. Case Report: We present a rare case of a 17 year-old male diagnosed with subcutaneous pannicultic T-cell lymphoma manifesting with prolonged fever, weight loss, and multiple subcutaneous nodules. He also presented with extra-cutaneous manifestations of facial edema, pleural effusion and ascites, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia. Conclusion and Summary: Subcutaneous Panniculitic T-Cell Lymphoma may present with an unusual finding of facial edema, ascites and pleural effusion, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia alongside skin manifestations of subcutaneous panniculitis. HPS may complicate the course, treatment decisions and outcome of the disease.